Delineating the Clinical Spectrum Associated With Xq25q26.2 Duplications: Report of 2 Families and Review of the Literature

J Child Neurol. 2019 Feb;34(2):86-93. doi: 10.1177/0883073818811454. Epub 2018 Nov 21.

Abstract

To date, 13 patients with interstitial microduplications involving Xq25q26.2 have been reported. Here, we report 6 additional patients from 2 families with duplications involving Xq25q26.2. Family I carries a 5.3-Mb duplication involving 26 genes. This duplication was identified in 3 patients and was associated with microcephaly, growth failure, developmental delay, and dysmorphic features. Family II carries an overlapping 791-kb duplication that involves 3 genes. This duplication was identified in 3 patients and was associated with learning disability and speech delay. The size and gene content of published overlapping Xq25q26.2 duplications vary, making it difficult to define a critical region or establish a genotype-phenotype correlation. However, patients with overlapping duplications have been found to share common clinical features including microcephaly, growth failure, intellectual disability, learning difficulties, and dysmorphic features. The 2 families presented here provide additional insight into the phenotypic spectrum and clinical significance of duplications in this region.

Keywords: Xq25q26 duplication; genomic microarray analysis; growth failure; intellectual disability; multiple congenital anomalies.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple / genetics*
  • Child
  • Child, Preschool
  • Chromosome Duplication*
  • Chromosomes, Human, X*
  • Developmental Disabilities / genetics*
  • Female
  • Humans
  • Intellectual Disability / genetics*
  • Language Development Disorders / genetics
  • Male
  • Microcephaly / genetics*
  • Pedigree
  • Phenotype*